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Mesenteric Lymphatic Malformation
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        • Diagnostic Checklist

          TERMINOLOGY

          • Synonyms

            • Mesenteric or omental cyst; lymphangioma (antiquated)
          • Definitions

            • Subtype of congenital slow-flow vascular malformation due to error of lymphatic vessel formation
            • Results in well-defined, cyst-like (macrocystic) &/or infiltrative, solid-appearing (microcystic) mass of abnormal lymphatic channels focally or diffusely within mesentery
              • Individual cyst size: Macrocyst > 1 cm, microcyst < 1 cm
            • Often lacks communication with normal lymphatics
            • Not neoplastic

          IMAGING

          • General Features

            • Radiographic Findings

              • Ultrasonographic Findings

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Dori Y et al: Intramesenteric dynamic contrast pediatric MR lymphangiography: initial experience and comparison with intranodal and intrahepatic MR lymphangiography. Eur Radiol. 30(10):5777-84, 2020
                                        2. Gasparella P et al: Giant lymphatic malformation causing abdominal compartment syndrome in a neonate: a rare surgical emergency. J Surg Case Rep. 2020(8):rjaa252, 2020
                                        3. Dong J et al: CT lymphangiography (CTL) in primary intestinal lymphangiectasia (pil): a comparative study with intraoperative enteroscopy (IOE). Acad Radiol. 26(2):275-81, 2019
                                        4. Prasad D et al: Clinical profile, response to therapy, and outcome of children with primary intestinal lymphangiectasia. Dig Dis. 37(6):458-66, 2019
                                        5. Clement C et al: An acute presentation of pediatric mesenteric lymphangioma: a case report and literature overview. Acta Chir Belg. 118(5):331-5, 2018
                                        6. ISSVA Classification of Vascular Anomalies. Published April 2014. Updated May 2018. Accessed March 20, 2021. http://issva.org/classification
                                        7. Dillman JR et al: Imaging of the pediatric peritoneum, mesentery and omentum. Pediatr Radiol. 47(8):987-1000, 2017
                                        8. Kim SH et al: Clinical features of mesenteric lymphatic malformation in children. J Pediatr Surg. 51(4):582-7, 2016
                                        9. Bagrodia N et al: Management of lymphatic malformations in children. Curr Opin Pediatr. 27(3):356-63, 2015
                                        10. Malone LJ et al: Pediatric lymphangiectasia: an imaging spectrum. Pediatr Radiol. 45(4):562-9, 2015
                                        11. Elluru RG et al: Lymphatic malformations: diagnosis and management. Semin Pediatr Surg. 23(4):178-85, 2014
                                        12. Russell KW et al: Sclerotherapy for intra-abdominal lymphatic malformations in children. Eur J Pediatr Surg. 24(4):317-21, 2014
                                        13. Trenor CC 3rd et al: Complex lymphatic anomalies. Semin Pediatr Surg. 23(4):186-90, 2014
                                        14. Cauley CE et al: Giant mesenteric lymphatic malformation presenting as small bowel volvulus. J Surg Case Rep. 2013(9), 2013
                                        15. Chang TS et al: Mesenteric cystic masses: a series of 21 pediatric cases and review of the literature. Fetal Pediatr Pathol. 30(1):40-4, 2011
                                        16. Chaudry G et al: Sclerotherapy of abdominal lymphatic malformations with doxycycline. J Vasc Interv Radiol. 22(10):1431-5, 2011
                                        17. Traubici J et al: Mesenteric lymphatic malformation associated with small-bowel volvulus - two cases and a review of the literature. Pediatr Radiol. 32(5):362-5, 2002
                                        18. Stoupis C et al: Bubbles in the belly: imaging of cystic mesenteric or omental masses. Radiographics. 14(4):729-37, 1994
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Clinical Issues

                                              • Diagnostic Checklist

                                                TERMINOLOGY

                                                • Synonyms

                                                  • Mesenteric or omental cyst; lymphangioma (antiquated)
                                                • Definitions

                                                  • Subtype of congenital slow-flow vascular malformation due to error of lymphatic vessel formation
                                                  • Results in well-defined, cyst-like (macrocystic) &/or infiltrative, solid-appearing (microcystic) mass of abnormal lymphatic channels focally or diffusely within mesentery
                                                    • Individual cyst size: Macrocyst > 1 cm, microcyst < 1 cm
                                                  • Often lacks communication with normal lymphatics
                                                  • Not neoplastic

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • Ultrasonographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Gross Pathologic & Surgical Features

                                                                  • Microscopic Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Dori Y et al: Intramesenteric dynamic contrast pediatric MR lymphangiography: initial experience and comparison with intranodal and intrahepatic MR lymphangiography. Eur Radiol. 30(10):5777-84, 2020
                                                                              2. Gasparella P et al: Giant lymphatic malformation causing abdominal compartment syndrome in a neonate: a rare surgical emergency. J Surg Case Rep. 2020(8):rjaa252, 2020
                                                                              3. Dong J et al: CT lymphangiography (CTL) in primary intestinal lymphangiectasia (pil): a comparative study with intraoperative enteroscopy (IOE). Acad Radiol. 26(2):275-81, 2019
                                                                              4. Prasad D et al: Clinical profile, response to therapy, and outcome of children with primary intestinal lymphangiectasia. Dig Dis. 37(6):458-66, 2019
                                                                              5. Clement C et al: An acute presentation of pediatric mesenteric lymphangioma: a case report and literature overview. Acta Chir Belg. 118(5):331-5, 2018
                                                                              6. ISSVA Classification of Vascular Anomalies. Published April 2014. Updated May 2018. Accessed March 20, 2021. http://issva.org/classification
                                                                              7. Dillman JR et al: Imaging of the pediatric peritoneum, mesentery and omentum. Pediatr Radiol. 47(8):987-1000, 2017
                                                                              8. Kim SH et al: Clinical features of mesenteric lymphatic malformation in children. J Pediatr Surg. 51(4):582-7, 2016
                                                                              9. Bagrodia N et al: Management of lymphatic malformations in children. Curr Opin Pediatr. 27(3):356-63, 2015
                                                                              10. Malone LJ et al: Pediatric lymphangiectasia: an imaging spectrum. Pediatr Radiol. 45(4):562-9, 2015
                                                                              11. Elluru RG et al: Lymphatic malformations: diagnosis and management. Semin Pediatr Surg. 23(4):178-85, 2014
                                                                              12. Russell KW et al: Sclerotherapy for intra-abdominal lymphatic malformations in children. Eur J Pediatr Surg. 24(4):317-21, 2014
                                                                              13. Trenor CC 3rd et al: Complex lymphatic anomalies. Semin Pediatr Surg. 23(4):186-90, 2014
                                                                              14. Cauley CE et al: Giant mesenteric lymphatic malformation presenting as small bowel volvulus. J Surg Case Rep. 2013(9), 2013
                                                                              15. Chang TS et al: Mesenteric cystic masses: a series of 21 pediatric cases and review of the literature. Fetal Pediatr Pathol. 30(1):40-4, 2011
                                                                              16. Chaudry G et al: Sclerotherapy of abdominal lymphatic malformations with doxycycline. J Vasc Interv Radiol. 22(10):1431-5, 2011
                                                                              17. Traubici J et al: Mesenteric lymphatic malformation associated with small-bowel volvulus - two cases and a review of the literature. Pediatr Radiol. 32(5):362-5, 2002
                                                                              18. Stoupis C et al: Bubbles in the belly: imaging of cystic mesenteric or omental masses. Radiographics. 14(4):729-37, 1994